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Tuberous Sclerosis Complex (TSC) is caused by mutations in  TSC1  or  TSC2 , which encode negative regulators of the mTOR signaling pathway. The renal abnormalities associated with TSC include angiomyolipoma, cysts, and renal cell carcinoma. Here we report that specific ablation of  Tsc1  using the mesenchymal stem cell-osteoblast lineage markers induced cystogenesis in mice. Using  Rosa-tdTomato  mice, we found that Prx1- or Dermo1-labeled cells were present in the nephron including glomerulus but they were not stained by markers for podocytes, mesangial cells, endothelial cells, or proximal or loop of Henle tubular cells, while Osx is known to label tubular cells.  Tsc1  deficiency in Prx1 lineage cells caused development of mild cysts that were positive only for Tamm-Horsfall protein (THP), a loop of Henle marker, while  Tsc1  deficiency in Osx lineage cells caused development of cysts that were positive for Villin, a proximal tubular cell marker. On the other hand,  Tsc1  deficiency in the Dermo1 lineage did not produce detectable phenotypical changes in the kidney. Cyst formation in  Prx1-Cre ;  Tsc1   f/f   and  Osx-Cre ;  Tsc1   f/f   mice were associated with increase in both proliferative and apoptotic cells in the affected tissue and were largely suppressed by rapamycin. These results suggest that Prx1 and Osx lineages cells may contribute to renal cystogenesis in TSC patients.

Tsc1 ablation in Prx1 and Osterix lineages causes renal cystogenesis in mouse